Table 1.
Finalized set of the quality indicators for the management and outcomes of adults with pulmonary arterial hypertension. (Originally derived from [7].)
| Domain | Content | Med | Min | Max | Mo |
|---|---|---|---|---|---|
| 1. Structural framework | |||||
| 1.1 | Pulmonary hypertension centers that have a specialized MDT responsible for the management of patients with PAH Note: MDT consists of at least a cardiologist, pulmonologist, and specialist nurse. Collaboration should be established with a rheumatologist, interventional radiologist, cardiothoracic surgeon, social worker, and psychologist |
9 | 8 | 9 | 9 |
| 1.2 | Pulmonary hypertension centers that have the following facilities and skills: | ||||
| - A ward where healthcare providers have expertise in PAH; | 9 | 8 | 9 | 9 | |
| - A specialist outpatient service; | 9 | 7 | 9 | 9 | |
| - An intermediate/intensive care unit; | 9 | 8 | 9 | 9 | |
| - A 24/7 emergency care; | 9 | 8 | 9 | 9 | |
| - An interventional radiology unit (for treatment of hemoptysis); | 8 | 7 | 8 | 8 | |
| - Diagnostic investigations, including echocardiography, CT scanning, nuclear medicine, MRI, exercise tests, and PFT; | 9 | 8 | 9 | 9 | |
| - A cardiac catheterization laboratory with vasodilator testing available; | 9 | 4 | 9 | 9 | |
| - Access to genetic counseling and testing; | 9 | 7 | 9 | 9 | |
| - Fast and easy access to cardiothoracic and vascular surgery, cardiac anesthesia, and ECMO; | 9 | 8 | 9 | 9 | |
| - Established collaboration with a lung/heart–lung transplantation center; | 9 | 7 | 9 | 9 | |
| 1.3 | Pulmonary hypertension centers that participate in a national or an international PAH registry | 9 | 7 | 9 | 9 |
| 1.4 | Pulmonary hypertension centers that have a fast-track policy to review urgent referrals within 1–2 weeks | 9 | 8 | 9 | 9 |
| 2. Diagnosis and risk stratification | |||||
| 2.1 | Proportion of patients with suspected PAH who undergo pulmonary function test (including lung volumes and DLCO) at the time of diagnostic work-up | 9 | 8 | 9 | 9 |
| 2.2 | Proportion of patients with suspected PAH who have an echocardiography at the time of diagnostic work-up | 9 | 9 | 9 | 9 |
| 2.3 | Proportion of patients with suspected PAH who have a RHC at the time of diagnostic work-up | 9 | 8 | 9 | 9 |
| 2.4 | Proportion of patients with suspected PAH who have perfusion imaging (V/Q scan or new modality) to exclude CTEPH at the time of diagnostic work-up Note: Alternative perfusion imaging techniques include iodine subtraction mapping, dual-energy CT, or MRI perfusion |
9 | 9 | 9 | 9 |
| 2.5 | Proportion of patients with suspected PAH who have been screened for CTD at the time of diagnostic work-up | 9 | 8 | 9 | 9 |
| 2.6 | Proportion of patients with a diagnosis of idiopathic, heritable, or drug-induced PAH who have RHC with acute vasodilator testing at the time of diagnostic work-up | 9 | 4 | 9 | 9 |
| 2.7 | Proportion of patients with a diagnosis of PAH who have their WHO-FC, NT-proBNP (or BNP) and 6MWT assessed at the time of PAH diagnosis | 9 | 8 | 9 | 9 |
| 2.8 | Proportion of patients with a diagnosis of PAH who have their risk assessed using a validated tool (e.g., ESC/ERS guidelines) at the time of PAH diagnosis | 9 | 6 | 9 | 9 |
| 2.9 | Proportion of patients with a diagnosis of PAH who have their quality of life assessed using a validated tool (e.g., Emphasis-10, SF-36, etc.) at the time of PAH diagnosis | 8 | 5 | 9 | 8 |
| Secondary 2 | Pulmonary hypertension centers that can perform exercise RHC in patients with suspected PAH at high risk at the time of diagnostic work-up | 8 | 4 | 9 | 8 |
| 3. Initial treatment | |||||
| 3.1 | Proportion of patients with a diagnosis of non-vasoreactive idiopathic, heritable, or drug-associated PAH and at high risk without significant cardiopulmonary comorbidities who are prescribed i.v./s.c. prostacyclin analogues | 9 | 8 | 9 | 9 |
| 3.2 | Proportion of patients with a diagnosis of non-vasoreactive idiopathic, heritable, drug-associated or CTD-associated PAH and at low or intermediate risk without significant cardiopulmonary comorbidities who are prescribed initial combination therapy with a NO donor and an ERA | 9 | 8 | 9 | 9 |
| 3.3 | Proportion of patients with a diagnosis of vasoreactive idiopathic, heritable, or drug-associated PAH and acute vasodilator response who are prescribed high doses of calcium channel blockers | 8 | 4 | 9 | 9 |
| 4. Follow-up | |||||
| 4.1 | Proportion of patients with a diagnosis of PAH who have their risk assessed using a validated tool (e.g., ESC/ERS guidelines) at least every 6 months | 9 | 6 | 9 | 9 |
| 4.2 | Proportion of patients with a diagnosis of PAH who have been informed about available patient association/support group(s) | 8 | 4 | 9 | 9 |
| 4.3 | Proportion of patients with a diagnosis of PAH who have their WHO-FC, NT-proBNP (or BNP) and 6MWT assessed at least every 6 months | 9 | 7 | 9 | 9 |
| 4.4 | Proportion of patients with a diagnosis of PAH in whom low risk is not achieved who have a discussion with a member of the MDT on treatment strategy | 8 | 7 | 9 | 9 |
| 4.5 | Proportion of patients with a diagnosis of PAH and at intermediate-high or high risk who are evaluated for lung transplantation Note: Who are eligible for lung transplantation (based on age and comorbidities) and have been established on a combination therapy. |
8 | 5 | 9 | 9 |
| 4.6 | Proportion of patients with a diagnosis of PAH who have not achieved low risk for whom regular hemodynamic assessment is considered at least every 12 months | 9 | 8 | 9 | 9 |
| Secondary 4 | Proportion of patients with a diagnosis of PAH who have their quality of life assessed using a validated tool at least every 6 months | 8 | 6 | 9 | 8 |
| 5. Outcomes | |||||
| 5.1 | Median time between establishing the diagnosis of PAH (i.e., date of diagnostic RHC) and commencing PAH therapy | 9 | 8 | 9 | 9 |
| 5.2 | Median time between referral and commencing PAH therapy Note: Referral time is date of receipt of the referral request by the specialist PAH center |
9 | 8 | 9 | 9 |
Med: median, Min: minimum, Max: maximum, Mo: mode, PAH: pulmonary arterial hypertension, MDT: multidisciplinary team, CT: computed tomography, MRI: magnetic resonance imaging, PFT: pulmonary function test, ECMO: extracorporeal membrane oxygenation, RHC: right heart catheterization, CTEPH: chronic thromboembolic pulmonary hypertension, DLCO: diffusing capacity of the lung for carbon monoxide, CTD: connective tissue disease, WHO-FC: World Health Organization functional class, NT-proBNP: N-terminal pro B-type natriuretic peptide, BNP: B-type natriuretic peptide, 6MWT: 6-Minute Walk Test, ESC/ERS: European Society of Cardiology/European Respiratory Society, SF-36, 36-Item Short Form Health Survey, i.v.: intravenous, s.c.: subcutaneous, NO: nitric oxide, ERA: endothelin receptor antagonist.